横纹肌样脑膜瘤的诊断与鉴别诊断

横纹肌样脑膜瘤(Rhabdoid meningioma,RM)是脑膜瘤的一种特殊类型,病理改变以大量横纹肌样肿瘤细胞伴或不伴有脑膜瘤形态为特点.由于该病很少见,且组织形态与多个肿瘤相似,因而易导致误诊和漏诊.现结合我科诊断的1例病例及文献对该病病理特点、免疫表型等进行分析.     

横纹肌样脑膜瘤临床病理观察

目的:探讨横纹肌样脑膜瘤(RM)的临床病理特点和鉴别诊断。方法:回顾分析4例RM的临床表现、组织形态学及免疫表型,并复习相关文献。结果:4例RM患者平均年龄20.5岁,男女各半。受累部位:颅内3例,椎管内1例。临床主要表现为颅压升高和神经系统压迫症状。光镜下肿瘤细胞呈横纹肌样特点,嗜酸性胞浆及偏位核。免疫组织化学染色显示肿瘤细胞EMA和Vim弥漫或灶状阳性,GFAP、HMB45和PR阴性。结论:横纹肌样脑膜瘤非常罕见,具有横纹肌样的细胞形态学特点,免疫组化是正确诊断横纹肌样脑膜瘤的重要依据。     

横纹肌样脑膜瘤1例并文献复习

横纹肌样脑膜瘤（rhahdoid meningioma, RM）是一种少见的高度恶性肿瘤，具有易复发、病程进展迅速的特点。由于该病罕见，极易误诊。为了更好地认识该病，现报道1例，并进行文献复习。     

朗格汉斯组织细胞增生症2例并文献复习

目的：探讨朗格汉斯组织细胞增生症的临床病理特点、鉴别诊断及生物学特征。方法：对2例朗格汉斯组织细胞增生症进行组织学观察和免疫组化染色，并复习相关文献。结果：两例患者分别为成人和儿童，均以头部肿物为首发症状，伴有不同程度的颅骨破坏。手术切除病灶后成人无复发，儿童一个月后颈部又发现肿物并侵犯横纹肌。2例均经病理学诊断为朗格汉斯组织细胞增生症。结论：朗格汉斯组织细胞增生症是一种影响网状内皮系统的疾病，主要以朗格汉斯细胞浸润为主，组织学为良性，但其生物学行为却呈侵袭性、破坏性，可累及骨、肺等多个器官。     

儿童肾上皮样血管平滑肌脂肪瘤1例及文献复习

目的：探讨肾上皮样血管平滑肌脂肪瘤（epithelioid angiomyolipoma,EAML）的临床特征、诊断、治疗与预后。方法：通过1例儿童肾 EAML 的报道,分析其临床资料并复习相关文献。结果：本例患儿女性,6岁,临床表现为腹部胀痛,左侧腹部可触及一巨大肿物。经肾肿瘤根治术,肉眼检查见肿瘤位于肾下极,大小约15cm ×10cm ×8cm,其包膜完整,与周围组织界限清楚。镜检示瘤体主要成分为上皮样细胞,核分裂象罕见,多核瘤巨细胞多见。免疫组化结果支持肾上皮样血管平滑肌脂肪瘤。术后随访6月,未见肿瘤复发。结论：肾 EAML 是一种罕见的肾脏间叶肿瘤,儿童病例报道甚少。临床表现缺乏特异性,诊断依靠病理学检查。手术切除为主要治疗方法,术后有复发和转移可能,宜严密随访。     

儿童涎腺恶性肌上皮瘤1例

患儿，男性。主因左颈部包块６年，３次手术切除３次复发，于１９９９年１２月２８日收入我科。１９９３年底无意中发现患儿左颈部黄豆大小包块，６个月后长至杏核大小，在当地县医院行包块切除术，病理诊断为“单形性腺瘤”。１９９５年５月第１次复发，即在当地行第２次包块切除术，术后病理为：“腺泡状横纹肌肉瘤”，但我院病理会诊诊断为“恶性肌上皮瘤”。光镜下见大量上皮性肿瘤细胞呈巢状排列，浆细胞样组织及胞浆空淡细胞，S１００染色阳性。遂行局部扩大术并全颈淋巴结清扫术。１９９６年７月第２次复发，再行包块切除术，术后病…     

左颞部肾外恶性横纹肌样瘤1例

1　病例介绍患儿 ,男 ,1岁 ,因左颞部肿物 1月余于 2 0 0 0年 11月 10日入住佛山市第一医院外科。查体 :左颞部皮下一肿物 ,直径约 3cm,无明显压痛 ,在左上颈部触及一肿大淋巴结约直径 1cm,质硬 ,ECT报告 :左侧颞部局部肿物软组织血运丰富 ,较对侧明显增加 ,提示血管瘤可能。 2 0 0 0年 11月 16日手术切除肿物 ,切开颞肌 ,可见一 2 cm肿物位于颞骨骨膜上 ,边缘部分长入颔骨骨缝中 ,质脆 ,切面鱼肉状 ,术后病理报告示 :左颞部肾外恶性横纹肌样瘤 ,于 2 0 0 0年 12月 4日转放疗科治疗。2　讨论恶性横纹肌样瘤 ,于 1978年国外首先报道 ,认为…     

儿童颅内脑膜瘤8例报告

目的 回顾分析我院收治的儿童脑膜瘤特点。方法 8例儿童颅内脑膜瘤均行手术治疗,辅助检查包括头颅平行、脑血管造影、CT、MR等,随访5例。结果 8例均达肿瘤全切,病理类型主要为上皮型及纤维型,5例随访2－5年未发现肿瘤复发。结论 儿童脑膜瘤少见,病理类型以上皮型及纤维型多见,肿瘤全切预后良好。     

WHOⅡ级脑膜瘤术后复发的相关性因素分析

目的:探讨WHOⅡ级脑膜瘤的手术治疗方式及影响患者术后复发的相关因素。方法:以我院2008年1月-2018年6月收治的103名WHOⅡ级的脑膜瘤患者为研究对象，103例WHOⅡ级脑膜瘤患者中9例采用肿瘤次全切（Simpson IV级），94例采用肿瘤全切术（Simpson I-III级）。103例患者术后复发31例，无复发72例。分析患者年龄、性别、脑膜瘤病理类型、瘤周水肿、肿瘤最大径、肿瘤切除程度及术后放疗等与肿瘤复发的关系。结果:单因素Cox回归分析显示，年龄、性别、肿瘤最大径与肿瘤病理类型对WHO Ⅱ级脑膜瘤术后复发影响较小（P＞0.05）。多因素Cox回归分析显示，伴有瘤周水肿、Simpson分级是影响WHO Ⅱ级脑膜瘤术后复发的独立危险因素（P＜0.05）。术后放疗是WHO Ⅱ级脑膜瘤术后复发的保护因素（P＜0.05）。结论:Simpson分级、有无瘤周水肿及术后是否放疗是影响WHO Ⅱ级脑膜瘤是否复发的相关因素。     

成人膀胱横纹肌肉瘤2例报告并文献复习

 目的探讨成人膀胱横纹肌肉瘤的病变特点及临床诊治方法。方法回顾性分析2例成人膀胱横纹肌肉瘤患者的临床资料。结果本组2例均经手术治疗，术后经免疫组化及病理检查证实为膀胱横纹肌肉瘤。术后2例患者均给予VAC方案规则化疗及放疗，恢复良好。其中例1随诊2年，未见复发；例2八个月后死于脑血管意外。结论成人膀胱横纹肌肉瘤属少见病，恶性程度高且症状不典型，确诊需要靠免疫组化及病理证实，以手术为主的综合治疗方式效果较好。     

Clinical Research of a Modified Midfacial Degloving in a Maxillectomy （with a Report of a Typical Case）

OBJECTIVE To investigate the feasibility of employing a modified midfacial degloving in maxillectomy. METHODS Eight patients with carcinoma of the maxillary sinus underwent a modified midfacial degloving operation.The tumors were classified according to the 2002 AJCC system.The TNM staging of the cases was as follows:1 T4aN0M0,2 T3N0M0 and 5 T2N0M0.Of the 8 cases,1 patient underwent extended maxillectomy;exenteration of the orbit;tumorectomy of the sphenomaxillary and infratemporal fossae.Two patients received a total maxillectomy,and 5 a partial resection of the maxilla. Postoperative pathological report:4 well-di?erentiated squamous carcinoma,2 moderately-differentiated squamous carcinoma,1 mucoepidermoid carcinoma and 1 adenoid cystic carcinoma. RESULTS A modified midfacial degloving operation can sufficiently expose a field of operation,resect the tumor within a safe margin,and leave no facial cicatricles.One patient died of intracranial metastasis 8 months a er operation.We observed no recurrences or metastasis in other patients during the period of follow-up. CONCLUSION The major advantages of employing the modified midfacial degloving in maxillectomy is that a facial incision can be avoided.It has an advantage of minimal invasive surgery.     

Circumcision: a review and a new technique

Numerous techniques for circumcision have been described in the literature. They range from the dorsal slit incision, the squeeze technique using the Gomco clamp or Plastibell, the sleeve resection technique, and the guillotine technique. An alternative technique, which has not been previously described, is presented.     

Melatonin as a radioprotective agent: a review

Melatonin (N-acetyl-5-methoxytryptamine), the chief secretory product of the pineal gland in the brain, is well known for its functional versatility. In hundreds of investigations, melatonin has been documented as a direct free radical scavenger and an indirect antioxidant, as well as an important immunomodulatory agent. The radical scavenging ability of melatonin is believed to work via electron donation to detoxify a variety of reactive oxygen and nitrogen species, including the highly toxic hydroxyl radical. It has long been recognized that the damaging effects of ionizing radiation are brought about by both direct and indirect mechanisms. The direct action produces disruption of sensitive molecules in the cells, whereas the indirect effects ( approximately 70%) result from its interaction with water molecules, which results in the production of highly reactive free radicals such as *OH, *H, and e(aq)- and their subsequent action on subcellular structures. The hydroxyl radical scavenging ability of melatonin was used as a rationale to determine its radioprotective efficiency. Indeed, the results from many in vitro and in vivo investigations have confirmed that melatonin protects mammalian cells from the toxic effects of ionizing radiation. Furthermore, several clinical reports indicate that melatonin administration, either alone or in combination with traditional radiotherapy, results in a favorable efficacy:toxicity ratio during the treatment of human cancers. This article reviews the literature from laboratory investigations that document the ability of melatonin to scavenge a variety of free radicals (including the hydroxyl radical induced by ionizing radiation) and summarizes the evidence that should be used to design larger translational research-based clinical trials using melatonin as a radioprotector and also in cancer radiotherapy. The potential use of melatonin for protecting individuals from radiation terrorism is also considered.     

How a bronchoscopy avoided a pediatric pneumonectomy!

Primary Lung pathology without any intraluminal lesion needs extensive lung resections but It is always necessary to rule out any intraluminal pathology before submitting the patient to a Pneumonectomy. The case described illustrates how a last minute, on the table Exploratory Bronchoscopy revealed a Broncholith which avoided an extensive surgery thus reducing the morbidity